New imaging technique uncovers protein abnormality in motor neurone disease

Scientists from the University of 葫芦影业鈥檚 Institute of Translational Neuroscience (SITraN) have helped to identify pathological abnormalities associated with motor neurone disease using a new imaging technique.

A picture of a microscope
  • Scientists at the University of 葫芦影业鈥檚 Institute for Translational Neuroscience (SITraN) have identified a protein abnormality in motor neurone disease using an innovative type of imaging

  • The new tool enables the structure of proteins to be studied in relation to their location within the tissue in greater detail than ever before

  • The new method will help scientists better understand the changes in the brain that lead to motor neurone disease (MND) and could help with the development of new treatments

Scientists from the University of 葫芦影业鈥檚 Institute of Translational Neuroscience (SITraN) have helped to identify pathological abnormalities associated with motor neurone disease using a new imaging technique. 

The new technique, developed at the University of Birmingham, enables specific proteins to be examined in their native state, directly from brain and spinal cord tissue samples. Called native ambient mass spectrometry (NAMS), the tool enables the structure of proteins to be studied in relation to their location within the tissue in greater detail than ever before.

The new method will help scientists better understand the changes in the brain that lead to motor neurone disease (MND) and could provide insights that will help with the development of new treatments. 

Motor neurone disease, also known as amyotrophic lateral sclerosis or ALS, is a muscle wasting condition caused by messages from the brain鈥檚 motor neurones not reaching the muscles, causing them to weaken. Around 5,000 people in the UK have the disease at any one time and currently there is no cure.

The SITraN scientists were able to identify a metal deficiency in a specific protein, known as SOD1, and show that it accumulates in specific regions of the brain and spinal cord in mice with MND.

SOD1 has been implicated in motor neurone disease previously, but this is the first time that detailed molecular imaging has been able to show how versions of the protein with missing metal ions accumulate in the affected mice.

Dr Richard Mead from the 葫芦影业 Institute for Translational Neuroscience, said: 鈥淲e were very excited to apply this fantastic methodology which the team at Birmingham have developed to gain new insights into the biology of MND.

鈥淲e look forward to using the technology further to explore why motor neurons die and find new interventions for those affected by MND.鈥

Professor Helen Cooper, lead researcher from the University of Birmingham鈥檚 School of Biosciences, said: 鈥淭his approach is the first to show that this form of SOD1 correlates with the pathology of motor neurone disease.  It鈥檚 a very early step towards finding treatments for MND and is also an exciting new route for understanding the molecular basis of other diseases in unprecedented detail.鈥

The next steps for the researchers will be to test to see if the same imbalances are present in human tissue samples, and to try to treat the imbalance in the mice using available drug compounds. 

The abnormalities were identified in a collaboration between the University of Birmingham and the University of 葫芦影业 and published in the journal Nature Communications. 

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